Meningeal meningiomatosis in a child: case report.

We report a child with multifocal meningeal lesions demonstrating mixed meningiomatous histologic features. This lesion appears to have evolved over seven years, starting with a brief episode of garbled speech, nausea, and headache at 4.5 years of age. The child was then asymptomatic until 9 when she presented with bilateral leg weakness, in addition to her prior presenting symptoms and communicating hydrocephalus. Meningeal biopsies of two lesions were performed 18 months later. Immunohistochemistry and electron microscopy were needed to substantiate the histologic diagnosis. Radiation therapy to the craniospinal axis and corticosteroids were of some benefit, but more aggressive therapeutic modalities became necessary. The nosology of this lesion is discussed.

Dandy-Walker syndrome: clinico-pathological features and re-evaluation of modes of treatment.

Eighteen cases of the Dandy-Walker syndrome are presented. The clinical manifestations are analyzed. Almost half of these children had associated congenital anomalies. Only four had the "characteristic" radiological findings. Direct surgical attack upon the cyst was not a successful form of treatment. Satisfactory treatment consisted of shunting of the ventricular system but the incidence of complications was high. There was a 27% mortality. The etiology and pathology are discussed.

Delayed-onset dystonia in patients with "static" encephalopathy.

Eight cases of persistent dystonia appearing one to 14 years after non-progressive cerebral insults are described. Five were due to perinatal anoxia, one to trauma, and two to cerebral infarction. This phenomemon of delayed-onset dystonia has not been described previously, although review of earlier literature reveals several probable examples. Delayed-onset dystonia due to perinatal anoxia is an important diagnostic alternative to dystonia musculorum deformans for dystonia occurring in childhood.

The impact of psychiatric intervention on patients with uncontrolled seizures.

There is much evidence that emotional stess can trigger both neurogenic and hysterical seizures in susceptible patients. We reviewed out experience with 37 patients whose seizures appeared to be precipitated at times by emotional stress and had not been controlled by anticonvulsant medication alone. Approximately 70 per cent of patients demonstrated substantial improvement in seizure control after psychiatric treatment and maintained this improvement during follow-up. The findings of this study suggest that patient characteristics associated with better prognosis include normal intelligence, partial (as opposed to generalized) neurogenic seizures, a diagnosis of hysterical seizures, a less severely abnormal EEG, and being hypnotizable. After psychiatric treatment, 32 per cent of patients had their anticonvulsant medication reduced and another 16 per cent had it discontinued.

Hypopigmented iris spot. An early sign of tuberous sclerosis.

Hypopigmented skin spots, resembling the mountain ash leaf, may represent the earliest sign in tuberous sclerosis. We examined two patients with hypopigmented iris spots who suffered from this systemic disease. These iris spots may be analogous to the skin lesions, which have decreased amount of melanin in the melanosomes.